An Infant With Fever, Hepatosplenomegaly, and Pancytopenia
An Infant With Fever, Hepatosplenomegaly, and Pancytopenia
A previously healthy 8-month-old white female was admitted with a history of fever for 3 weeks. She had daily temperatures as high as 39.4°C associated with decreased activity. A mild nonproductive cough without rhinorrhea was noted by the mother during the first week of illness. There was no history of vomiting, diarrhea, rash, hand swelling, or redness of the eyes. Her appetite remained normal throughout the illness.
The infant was initially diagnosed with a viral illness by her physician, but because of persistent fever, she was subsequently admitted to a community hospital 1 week after the onset of her illness. Her evaluation there included chest radiography, complete blood counts, urine culture, and blood culture. She was discharged home after 2 days when all of the test results were negative. Two weeks later, she was brought to the emergency department at Children's Hospital of Michigan because of persistent fever, and was again admitted.
The patient had no significant past medical history. Her growth and development were appropriate for her age. Her immunizations were complete for her age. She lived with her parents in Detroit and had never traveled outside Michigan. There were no pets at home, and she did not attend day care.
Upon admission the infant was alert, interactive, and in no distress. She had a temperature of 40°C, pulse of 150 beats/min, respiratory rate of 32 breaths/min, blood pressure of 90/69 mm Hg, and oxygen saturation of 100% in room air. Abdominal examination revealed an enlarged spleen and liver that were palpable 7 cm and 4 cm below the costal margin, respectively. The remainder of the physical examination was normal.
The patient's white blood cell count was 6300/mm with 15% neutrophils, 76% lymphocytes, 8% monocytes, and 1% eosinophils. The hemoglobin was 11 g/dL, and the platelet count was 150,000/mm; the peripheral blood smear showed no cellular atypia. A repeat CBC on the next day showed white blood cell count of 4000/mm with 23% neutrophils, 63% lymphocytes, and 14% monocytes. The hemoglobin decreased to 9.8 g/dL, and the platelet count decreased to 88,000/mm. Aspartate aminotransferase and alanine aminotransferase were 162 and 163 units/L, respectively. Alkaline phosphotase was 152 units/L, serum albumin was 2.9 g/dL, lactate dehydrogenase was 357 units/L, serum uric acid level was 2.9 mg/dL, and ferritin level was 180 ng/mL. Epstein-Barr virus, cytomegalovirus, toxoplasma, and parvovirus serologies were negative. In addition, rapid plasma reagent and human immunodeficiency virus antibody by enzyme-linked immunosorbent assay were negative. A tuberculin skin test had 0 mm of induration.
Chest radiograph was normal, but abdominal ultrasonography confirmed the presence of massive hepatosplenomegaly without any identifiable intraabdominal mass. In light of her fevers, hepatosplenomegaly, and pancytopenia, the hematology-oncology service was consulted. A bone marrow aspiration was performed; the bone marrow smear examination and additional laboratory tests confirmed the diagnosis.
A previously healthy 8-month-old white female was admitted with a history of fever for 3 weeks. She had daily temperatures as high as 39.4°C associated with decreased activity. A mild nonproductive cough without rhinorrhea was noted by the mother during the first week of illness. There was no history of vomiting, diarrhea, rash, hand swelling, or redness of the eyes. Her appetite remained normal throughout the illness.
The infant was initially diagnosed with a viral illness by her physician, but because of persistent fever, she was subsequently admitted to a community hospital 1 week after the onset of her illness. Her evaluation there included chest radiography, complete blood counts, urine culture, and blood culture. She was discharged home after 2 days when all of the test results were negative. Two weeks later, she was brought to the emergency department at Children's Hospital of Michigan because of persistent fever, and was again admitted.
The patient had no significant past medical history. Her growth and development were appropriate for her age. Her immunizations were complete for her age. She lived with her parents in Detroit and had never traveled outside Michigan. There were no pets at home, and she did not attend day care.
Upon admission the infant was alert, interactive, and in no distress. She had a temperature of 40°C, pulse of 150 beats/min, respiratory rate of 32 breaths/min, blood pressure of 90/69 mm Hg, and oxygen saturation of 100% in room air. Abdominal examination revealed an enlarged spleen and liver that were palpable 7 cm and 4 cm below the costal margin, respectively. The remainder of the physical examination was normal.
The patient's white blood cell count was 6300/mm with 15% neutrophils, 76% lymphocytes, 8% monocytes, and 1% eosinophils. The hemoglobin was 11 g/dL, and the platelet count was 150,000/mm; the peripheral blood smear showed no cellular atypia. A repeat CBC on the next day showed white blood cell count of 4000/mm with 23% neutrophils, 63% lymphocytes, and 14% monocytes. The hemoglobin decreased to 9.8 g/dL, and the platelet count decreased to 88,000/mm. Aspartate aminotransferase and alanine aminotransferase were 162 and 163 units/L, respectively. Alkaline phosphotase was 152 units/L, serum albumin was 2.9 g/dL, lactate dehydrogenase was 357 units/L, serum uric acid level was 2.9 mg/dL, and ferritin level was 180 ng/mL. Epstein-Barr virus, cytomegalovirus, toxoplasma, and parvovirus serologies were negative. In addition, rapid plasma reagent and human immunodeficiency virus antibody by enzyme-linked immunosorbent assay were negative. A tuberculin skin test had 0 mm of induration.
Chest radiograph was normal, but abdominal ultrasonography confirmed the presence of massive hepatosplenomegaly without any identifiable intraabdominal mass. In light of her fevers, hepatosplenomegaly, and pancytopenia, the hematology-oncology service was consulted. A bone marrow aspiration was performed; the bone marrow smear examination and additional laboratory tests confirmed the diagnosis.
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